Asymmetric Dimethylarginine Levels and Its Correlation to Cerebral Blood Flow in Children with Sickle Cell Anemia

AbstractAsymmetric dimethylarginine (ADMA) level may play a role in the pathogenesis of cerebrovascular stroke in Children with Sickle Cell Anemia (SCA). To assess the plasma level of ADMA in children with SCA and its correlation to cerebral blood flow. This is a cross sectional study was carried out on 30 children with homozygous SCA under follow up in the Out Patients Clinic, Pediatric Department at Tanta University Hospital and 30 healthy children as a control group. Both groups had undergone the following investigations: Complete blood count, lactate dehydrogenase enzyme, and plasma level of ADMA by a commercial ADMA ELISA Kit. Trans-cranial Doppler were done for both groups. ADMA plasma level was significantly higher in-patient group in comparison to the control group (p 
Source: Indian Journal of Hematology and Blood Transfusion - Category: Hematology Source Type: research

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Conclusion: Our findings suggest that hydroxyurea is safe and may prevent silent stroke and stroke in sickle cell disease. More high-quality studies including RCTs are needed.
Source: Medicine - Category: Internal Medicine Tags: Research Article: Systematic Review and Meta-Analysis Source Type: research
Sickle cell disease (SCD) has evolved from a life-threatening condition in early childhood to a chronic disease in adults [1,2]. Improved supportive care [3], use of hydroxyurea therapy [4], and regular blood transfusion therapy for primary [5] and secondary [6] stroke prevention have resulted in improved childhood clinical outcomes and survival. Approximately 99% of all children born with SCD are expected to live to adulthood [7,8]. The most feared complication in children with SCD is stroke [9].
Source: Biology of Blood and Marrow Transplantation - Category: Hematology Authors: Source Type: research
Sickle cell disease (SCD) has evolved from a life-threatening condition in early childhood, to a chronic disease in adults [1,2]. Improved supportive care [3], use of hydroxyurea therapy [4], and regular blood transfusion therapy for primary [5] and secondary [6] stroke prevention, have resulted in improved childhood clinical outcomes and survival. Approximately 99% of all children born in high resource countries with SCD are expected to live to adulthood [7,8]. The most feared complication in children with SCD is stroke [9].
Source: Biology of Blood and Marrow Transplantation - Category: Hematology Authors: Source Type: research
The objective of this report is to describe the potential development of an extracellular superoxide dismutase-mimetic (SOD3-mimetic) to relieve sickle cell disease (SCD) children susceptible to stroke from being transfusion-dependent in order to avoid stroke. SanFlow [aka polynitroxylated pegylated hemoglobin (PNPH)], a SOD3-mimetic has been evaluated and approved by the FDA for further development as drug for the treatment of traumatic brain injury (TBI) complicated by hemorrhagic shock (HS), stroke, and SCD. PK&PD studies demonstrated that SanFlow works as a macromolecular SOD3-mimetic by protecting the endogenous vascu...
Source: Blood - Category: Hematology Authors: Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Poster II Source Type: research
Introduction. Despite the increasing of number of patients with Sickle Cell Disease (SCD) in Italy, due to multi-ethnic migratory phenomena, a large percentage of Caucasian sickle population is already present in Italy mainly with b-thal/HbS genotype. Red cell transfusion is one effective treatment for both acute and chronic complications of SCD, while hydroxycarbamide (HC) is used to reduce the frequency of painful vaso-occlusive crises (VOCs) and decrease the need for blood transfusion.Through the National Comprehensive Reference Centers for SCD, the Italian Society of Thalassemia and Hemoglobinopathies (SITE), in collab...
Source: Blood - Category: Hematology Authors: Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II Source Type: research
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Source: American Journal of Hematology - Category: Hematology Authors: Tags: Research Article Source Type: research
Blood transfusions are the mainstay of stroke prevention in pediatric sickle cell anemia (SCA), but the physiology conferring this benefit is unclear. Cerebral blood flow (CBF) and oxygen extraction fraction (OEF) are elevated in SCA, likely compensating for reduced arterial oxygen content (CaO2). We hypothesized that exchange transfusions would decrease CBF and OEF by increasing CaO2, thereby relieving cerebral oxygen metabolic stress. Twenty-one children with SCA receiving chronic transfusion therapy (CTT) underwent magnetic resonance imaging before and after exchange transfusions. Arterial spin labeling and asymmetric s...
Source: Blood - Category: Hematology Authors: Tags: Pediatric Hematology, Sickle Cell Disease, Transfusion Medicine, Free Research Articles, Red Cells, Iron, and Erythropoiesis Source Type: research
ConclusionsFVL was more prevalent among SS patients compared to controls and it was associated with higher incidence of disease complications among SCD patients.
Source: BMC Hematology - Category: Hematology Source Type: research
ré C Abstract Cerebral vasculopathy is a common and severe complication of sickle cell disease in children. The pathophysiology consists of progressive damage to the basal intracranial arteries and cerebral microcirculation, while chronic anemia worsens exposure to cerebral hypoxia. It results in stroke and subclinical or poorly symptomatic ischemic lesions. Many clinical, biological, and radiological risk factors have been identified. The prevention strategy through systematic transcranial Doppler screening of large-vessel vasculopathy has revolutionized the management of this disease and has greatly decre...
Source: Archives de Pediatrie - Category: Pediatrics Authors: Tags: Arch Pediatr Source Type: research
Background: Foetal haemoglobin (HbF) is a major modifying factor influencing sickle cell disease (SCD) severity. Despite this, HbF estimation is not routinely done in Nigeria. Relationship between HbF and SCD severity among affected children is also poorly studied.Methods: In this descriptive cross-sectional study, we determined the relationship between steady state HbF levels and disease severity of Nigerian children aged 1 – 15 years with homozygous SCD. For each child, the socio-demographic characteristics and SCD clinical severity were determined. The latter was assessed based on the frequency of significant pain...
Source: Mediterranean Journal of Hematology and Infectious Diseases - Category: Hematology Source Type: research
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